2uuu

File:2uuu.gif

spinqualitylabelsall models 2uuu, resolution 1.95Å Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D

ALKYLDIHYDROXYACETONEPHOSPHATE SYNTHASE IN P212121

Overview

Ether phospholipids are essential constituents of eukaryotic cell, membranes. Rhizomelic chondrodysplasia punctata type 3 is a severe, peroxisomal disorder caused by inborn deficiency of, alkyldihydroxyacetonephosphate synthase (ADPS). The enzyme carries out the, most characteristic step in ether phospholipid biosynthesis: formation of, the ether bond. The crystal structure of ADPS from Dictyostelium, discoideum shows a fatty-alcohol molecule bound in a narrow hydrophobic, tunnel, specific for aliphatic chains of 16 carbons. Access to the tunnel, is controlled by a flexible loop and a gating helix at the, protein-membrane interface. Structural and mutagenesis investigations, identify a cluster of hydrophilic catalytic residues, including an, essential tyrosine, possibly involved in substrate proton abstraction, and, the arginine that is mutated in ADPS-deficient patients. We propose that, ether bond formation might be orchestrated through a covalent imine, intermediate with the flavin, accounting for the noncanonical employment, of a flavin cofactor in a nonredox reaction.

About this Structure

2UUU is a Single protein structure of sequence from Dictyostelium discoideum with FAD and PL3 as ligands. Active as Alkylglycerone-phosphate synthase, with EC number 2.5.1.26 Known structural/functional Site: AC1. Full crystallographic information is available from OCA.

Reference

The crucial step in ether phospholipid biosynthesis: structural basis of a noncanonical reaction associated with a peroxisomal disorder., Razeto A, Mattiroli F, Carpanelli E, Aliverti A, Pandini V, Coda A, Mattevi A, Structure. 2007 Jun;15(6):683-92. PMID:17562315

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